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特发性嗜酸性粒细胞增多综合征表现为心脏压塞和多器官功能障碍。

Idiopathic hypereosinophilic syndrome presenting as cardiac tamponade and multiorgan dysfunction.

机构信息

Internal Medicine, Carle Foundation Hospital, Urbana, Illinois, USA.

Internal Medicine, Carle Foundation Hospital, Urbana, Illinois, USA

出版信息

BMJ Case Rep. 2023 Aug 30;16(8):e256274. doi: 10.1136/bcr-2023-256274.

Abstract

Idiopathic hypereosinophilic syndrome is characterised by the overproduction of eosinophils with tissue infiltration, leading to multiorgan dysfunction. Its heterogenous presentation makes the diagnosis challenging and easy to miss. A woman in her 70s was admitted with chest pain and shortness of breath. Diagnostic testing showed elevated cardiac enzymes, an ejection fraction of 45% and pericardial effusion. Pericardiocentesis helped her symptoms significantly. Cardiac catheterisation revealed patent coronary arteries. She was diagnosed with myopericarditis and discharged on non-steroidal anti-inflammatory drugs. She returned the following week with worsening chest pain, dyspnoea and diarrhoea. Chest imaging showed bilateral infiltrates. Diagnostic testing showed eosinophilic predominance in peripheral blood (59%), pericardial fluid (37%) and bronchoalveolar lavage (31%). After a negative infectious workup, she was started on glucocorticoids and responded favourably. She was discharged on steroids. Mepolizumab was initiated outpatient, and steroids were discontinued. Mepolizumab was discontinued after 2 years while monitoring her symptoms and eosinophil counts.

摘要

特发性嗜酸性粒细胞增多综合征的特征是嗜酸性粒细胞过度生成并浸润组织,导致多器官功能障碍。其表现多样,诊断具有挑战性且容易漏诊。一位 70 多岁的女性因胸痛和呼吸急促入院。诊断性检查显示心肌酶升高,射血分数为 45%,有心包积液。心包穿刺术显著缓解了她的症状。心导管检查显示冠状动脉通畅。她被诊断为心肌炎,并接受非甾体抗炎药治疗后出院。一周后她因胸痛、呼吸困难和腹泻再次入院。胸部影像学显示双侧浸润。诊断性检查显示外周血(59%)、心包液(37%)和支气管肺泡灌洗液(31%)中嗜酸性粒细胞占优势。在排除感染性疾病后,她开始接受糖皮质激素治疗,并取得了良好的疗效。她出院时接受类固醇治疗。美泊利珠单抗开始在门诊使用,随后停用了类固醇。在监测症状和嗜酸性粒细胞计数 2 年后停用美泊利珠单抗。

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本文引用的文献

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Eosinophilic Myocarditis: Classic Presentation of Elusive Disease.嗜酸性粒细胞性心肌炎:隐匿性疾病的典型表现。
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Biologic Agents for the Treatment of Hypereosinophilic Syndromes.治疗嗜酸性粒细胞增多综合征的生物制剂。
J Allergy Clin Immunol Pract. 2017 Nov-Dec;5(6):1502-1509. doi: 10.1016/j.jaip.2017.08.001.
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How I treat hypereosinophilic syndromes.我如何治疗高嗜酸性粒细胞综合征。
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